ACHD Care Guidelines

ACHA endorses the findings of the American College of Cardiology/American Heart Association 2008 Guidelines for the Management of Adults with Congenital Heart Disease, as well as ACC's 32nd Bethesda Conference Report.

The ACC/AHA Guidelines state that all ACHD patients should be seen once at an ACHD center once to confirm diagnosis. The document also includes defect-by-defect guidelines. For a PDF of the Guidelines, click here.

The Bethesda Report states that all adults born with complex congenital heart defects should be seen at specialized ACHD care centers. It also outlines the many unmet health care needs facing the ACHD community. For a PDF of the summary, click here.

The 32nd Bethesda Conference Guidelines—Highlights for the Patient

Introduction Every year, the American College of Cardiology (ACC) convenes a meeting in Bethesda, Maryland, to address an important issue in cardiology. In 2000, the 32nd Bethesda Conference gathered international congenital cardiology experts to address the needs of adults living with heart defects. Below is a summary of these expert's recommendations. You can read the full text here.

Task Force 1: The Changing Profile of Congenital Heart Disease in Adult Life

There are more adults with congenital heart defects than ever before. This is due in part to new and improved surgeries. There are an estimated 750,000 adults with congenital heart disease (CHD) in the United States. More than half need on-going specialized care.

Studies on children show that death rates from CHD surgeries are higher at centers that do not regularly do CHD operations. This is likely to apply to surgeries in adults.

There are many diagnostic tools for CHD. These include catherization, echocardiography, CT scans, and cardiac MRI. One study found that 53% of CHD echocardiograms done at local centers had errors. ACHD patients should get these studies done at centers that specialize in CHD.

Adults with congenital heart defects (ACHDers) need help switching from pediatric to adult care. A regional ACHD center that can meet patients' needs should be identified (see task force 4). This center should offer the same quality or higher quality care than the pediatric center.

Many factors can prevent adults with CHD from continuing their cardiac care. These include lack of symptoms and the idea that their CHD is "cured." Some ACHDers are attached to their existing centers and do not want to make a change.

All ACHD patients need education on their condition. Topics should include:

Name and details of their heart condition;

Surgeries that have been performed;

Frequency of follow-up;

Medications and side effects;

Preventing heart infections (endocarditis);

Exercise;

Birth control and family planning;

Career planning and resources;

Insurance;

Dental care;

Symptoms to look for;

When to contact their health care provider.

Patients should keep a copy of their health information. This should include test results and information on operations. A shorter "health passport" should be developed. This passport should be taken along when the patient travels.

Most adults with CHD can work. Adolescents with CHD should receive appropriate career counseling. Most should be encouraged to pursue higher education. Guidance should be given on any physical limits that may interfere with job choices.

Cyanotic ("blue") patients have many special health needs. Problems can occur throughout the body, not just in the heart and lungs. Primary care doctors should be made aware of the special needs of these patients. They should be seen at ACHD centers regularly. Phlebotomy (bloodletting), when appropriate, should only be done by ACHD experts.

All kinds of surgery can present risks for the CHD patient. This includes minor and routine procedures. Special attention is needed with anesthesia and to prevent heart infections (endocarditis). Patients on blood thinners also require special care. Ideally all surgery on these patients should be done at ACHD centers.

Cyanotic patients must have their IV lines kept free of air bubbles.

Contraception and Pregnancy

Women with CHD need to understand their risks from pregnancy. They should also understand any risks to their babies. All female ACHD patients should be educated about their birth control choices.

Sometimes sterilization is considered due to high risk. Patients should be given all the information they need to make this decision. This includes the possible risks of the sterilization procedure. Information about future advances that might make pregnancy possible should also be included.

Most female ACHD patients can have successful pregnancies. Those at higher risk should be seen at a center that specializes in high-risk pregnancy.

All ACHD patients should be offered genetic counseling. The chance of having a baby with CHD varies widely based on defect. All women with CHD should be offered fetal echocardiography between their 16th and 18th week of pregnancy.

Exercise

Studies on children suggest that exercise can be helpful for individuals with CHD. Exercise also benefits adults living with many other kinds of heart problems.

Studies suggest that most ACHD patients have reduced ability to exercise. This includes patients with repaired simple defects.

More studies are needed on the role of exercise in ACHD patients.

Psychosocial Issues

Adults with complex CHD can face many challenges to their mental health. Some live with the knowledge that they face a significant risk of dying young. Their health can limit their education, social life, activity level, and/or work life. Some face challenges with their appearance, such as scarring, small body size, blue lips, and finger clubbing.

CHD can affect lifestyle decisions, such as whether or not to leave home or marry. Some people may not have the information they need to make good choices in these areas. It is often difficult for people to discuss their CHD with their friends and family. Counseling should be available to help people address these issues.

More studies should be done on the emotional health of adults with CHD. Depression and anxiety have been shown to raise death rates in patients with other forms of heart disease.

Individuals with CHD may have had bad experiences with medical care. As children some experienced fear and untreated pain. These experiences can affect their ability to cope in a medical setting. Resources should be offered to help address these issues. These should include education on pain management.

Taking care of the emotional health of adults with CHD should be a priority.

Right now very few doctors have specific training in ACHD. Most ACHDers see adult cardiologists with little training in CHD. Others see pediatric cardiologists with little experience managing adult care.

More programs should be started to train health care workers in all aspects of ACHD care. The use of physician assistants and nurse practitioners may help to increase ACHD services.

Our health care system is not currently meeting the needs of adults living with heart defects.

Regional ACHD centers should be established. There should be one regional ACHD center for every 5 to 10 million people. This means 30 to 50 ACHD centers should be developed throughout the U.S.

Every adult with CHD should be evaluated at least once at an ACHD center. Ideally this includes those with "simple" heart defects (see chart).

Individuals with more complex defects should be seen regularly at an ACHD center. Frequency of follow up depends on their specific condition.

The role of the regional ACHD will vary based on the patient's needs. ACHD patients may have both a local cardiologist and ACHD specialists. The patient should also have a primary care doctor. The ACHD center should provide expert advice and information to the other health care providers.

A regional ACHD center should offer a team of specialized staff. This should include an ACHD cardiologist, other cardiologists, nurse practitioners and/or physician assistants, congenital heart surgeons and others. The team should work together to care for the patients.

Regional ACHD Centers should provide:

Patient counseling;

Operating rooms—specially equipped with personnel familiar with CHD surgery, fully trained congenital cardiac perfusionists, as well as a full array of diagnostic equipment;

Many ACHD patients have difficulty obtaining health insurance. Getting life insurance can also be a problem. Cardiologists should be willing to write letters describing conditions and long-term expectations.

Insurance companies need to be made aware of the special needs of ACHD patients.

Organized, effective and passionate advocacy for ACHD patients is needed. Patient groups and health care providers can work together for change.

The full text of the 32nd Bethesda Guidelines can be found here and in print: Care of the Adult With Congenital Heart Disease. Presented at the 32nd Bethesda Conference, Bethesda, Maryland, October 2-3, 2000. J Am Coll Cardiol 2001; 37: 1161-98. All references in this document can be found in the 32nd Bethesda Guidelines.

This document was prepared by the Medical Advisory Committee of the Adult Congenital Heart Association (ACHA), and reviewed by members of ACHA's Medical Advisory Board. Special thanks to Beth Adams and Amy Verstappen. All comments should be directed to info@achaheart.org.